A 5-year-old female presented a 4-year history of generalized well-demarcated asymptomatic brown to dark brown thin verrucous plaques with a Blaschkoid distribution. Histopathology was consistent with an epidermal nevus. Patient was diagnosed to have systematized verrucous epidermal nevus. Due to the extent of the lesions, surgical management was not feasible. Hence acitretin was given which showed partial decrease in the thickness of the lesions.

Introduction: Schimmelpenning syndrome may encompass abnormalities of the cardiovascular, skeletal, ophthalmologic and urogenital systems. Nevus sebaceous is a hallmark finding and ophthalmologic findings are seen in 59% of the cases which include colobomas and choristomas. Case Summary: A 1-month-old female presented with a verrucous plaque over the scalp and right zygomatic area upon birth. Physical examination reveals a linear yellowish alopecic verrucous plaque over the right frontal region, yellowish alopecic verrucous plaque topped with a skin colored papule over the right zygomatic region, conjunctival mass over the right eye and an atrophic patch with areas of circular erosion over the right occipital region. Ballard score and reflexes were appropriate for gestational age. Newborn screening was normal and otoacoustic-emission-test revealed no hearing loss. She was referred to an ophthalmologist and was assessed to have a lipodermoid, right upper eyelid and optic nerve coloboma. Cranial CT scan is unremarkable. Histopathology showed an increase in number of sebaceous glands with malformed hair units. She was managed holistically and does not have seizures and no secondary development of tumors in the nevus sebaceous. Conclusion Schimmelpenning syndrome is usually associated with the clinical triad of nevus sebaceous, mental retardation and seizures. In this case, seizures were absent, however, there is an associated lipodermoid, right upper eyelid and right optic nerve coloboma. In addition, she also presented with aplasia cutis congenita. Hence, it is important to look for other manifestations when patients present with nevus sebaceous because management requires collaboration with different specialties.
Nevus, Sebaceous of Jadassohn

No abstract available.
Carcinoma, Basal Cell* ; Hamartoma ; Nevus* ; Nevus, Sebaceous of Jadassohn

Inflammatory linear verrucous epidermal nevus (ILVEN) is an uncommon variant of verrucous epidermal nevus, clinically characterized by erythematous and hyperkeratotic verrucous papules with a typical linear arrangement. Most reported cases present as solitary lesions, but systematized ILVEN is rare. Epidermal nevus syndrome is a congenital disorder in which epidermal nevi are associated with abnormalities in other organ systems. However, ILVEN has been reported rarely in association with such abnormalities, including those of the skeleton. Here we report a rare case of systematized ILVEN presenting with concomitant mandibular hypoplasia.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Nevus ; Nevus, Sebaceous of Jadassohn* ; Skeleton

Adult ; Diagnosis, Differential ; Female ; Humans ; Nevus, Sebaceous of Jadassohn ; diagnosis ; pathology ; Psoriasis ; diagnosis ; pathology

Epidermal nevus syndromes are a group of congenital syndromes comprising epidermal nevi in conjunction with the central nervous system, ocular, musculoskeletal, and other organ anomalies. Skeletal manifestations in epidermal nevus syndromes are common, ranging from 50% to 66% of patients. However, fibrous dysplasia of the bone has been rarely described in association with epidermal nevus syndromes. Herein, we report a 46-year-old-male patient who presented with extensive epidermal nevi associated with monostotic fibrous dysplasia of the rib which is located ipsilateral to the skin findings.
Central Nervous System ; Fibrous Dysplasia, Monostotic ; Humans ; Nevus ; Nevus, Sebaceous of Jadassohn ; Ribs ; Skin

Nevus sebaceus of Jadassohn is a congenital cutaneous hamartoma comprised of multiple skin structures. It has the potential to develop into variety of neoplasms of various epidermal adnexal origins. While multiple tumors may occasionally arise, it is unusual for more than four tumors to arise simultaneously within a single sebaceus nevus. Here in, we report a case of a 70-year-old woman with six neoplastic proliferations including a syringocystadenoma papilliferum, pigmented trichoblastoma, tubular apocrine adenoma, sebaceoma, tumors of follicular infundibulum and superficial epithelioma with sebaceus differentiation arising in a long standing nevus sebaceus on the scalp. Our case is extraordinary because a single nevus sebaceus contained six neoplastic proliferations with differentiation toward the folliculosebaceous-apocrine unit.
Adenoma ; Aged ; Carcinoma ; Female ; Hamartoma ; Humans ; Nevus* ; Nevus, Sebaceous of Jadassohn ; Scalp ; Skin

Inflammatory linear verrucous epidermal nevi (ILVEN) were first described in the literature, in 1971, by Altman and Mehregan. Most cases were reported as solitary lesions. In contrast, systematized ILVEN, involving wide areas of the integument, has only rarely been reported. A variety of treatment modalities has been reported, ranging from topical medications, such as potent corticosteroids or tretinoin 0.1% to variable procedures, including CO2 and pulsed dye laser, cryotherapy, and surgical excision. However, multifocal skin lesion is more difficult to treat. An 8-year old boy was presented with an extensive thick scaly plaques and patches, affecting the trunk and the four extremities sparing face. It developed when he was 3 years old, and he complained pruritus. On physical examination, linear, or whirl-like scaly plaques were seen, along with Blaschko lines. Pathologic finding was in accordance to ILVEN. We treated him with topical calcipotriol, tacrolimus and systemic acitretin. After 8 months, the lesion and symptoms improved.
Acitretin ; Adrenal Cortex Hormones ; Calcitriol ; Cryotherapy ; Extremities ; Lasers, Dye ; Nevus ; Nevus, Sebaceous of Jadassohn ; Physical Examination ; Pruritus ; Skin ; Tacrolimus ; Tretinoin

Hemimegalencephaly is a rare congenital malformation of the cortical development arising from abnormal proliferation of anomalous neuronal and glial cells. The characteristic clinical manifestations are macrocephaly, psycomotor retardation, intractable seizure and hemihypertrophy of face, body and extremities, but musculoskeletal deformities are reported only in case of epidermal nevus syndrome. We report a case of hemimegalencephaly that was associated with foot deformity, without symptoms and signs of epidermal nevus syndrome.
Congenital Abnormalities ; Extremities ; Foot Deformities ; Foot Deformities, Congenital ; Macrocephaly ; Malformations of Cortical Development ; Neuroglia ; Neurons ; Nevus ; Nevus, Sebaceous of Jadassohn ; Seizures

During normal development of skin, pluripotential cells give rise to keratinocytes, sebaceus glands, hair follicles, apocrine glands, and eccrine glands. In epidermal nevi, these components emerge in an abnormal mixture within a circumscribed site. Many authors have categorized epidermal nevi according to their predominant component, however, there is often notable overlap which occurs within a single area or within contiguous areas. An 11-year-old female patient presented with linearly-arranged, multiple yellowish papules and a dark-brown colored verrucous plaque on the left occipital area, which had been present since birth. From the histopathological findings, the papules were diagnosed as nevus sebaceus and the plaque was diagnosed as verrucous epidermal nevus. Our present case gives support to the view that epidermal nevi have a spectrum of manifestations, including verrucous epidermal nevi and nevus sebaceus. To the best of our knowledge, this is the first report of a verrucous epidermal nevus contiguous to a nevus sebaceus in the Korean dermatological literature.
Apocrine Glands ; Child ; Eccrine Glands ; Female ; Hair Follicle ; Humans ; Keratinocytes ; Nevus* ; Nevus, Sebaceous of Jadassohn* ; Parturition ; Skin